Abstrakt
Idiopathic pulmonary fibrosis (IPF) is one of the most severe respiratory diseases with median survival 2.5 to 3 years if untreated. In the last 20 years enormous effort was focused to the area of research of pathogenesis and treatment of this disease.
Development and launch of the first antifibrotic drug, pirfenidone, in 2011 became a historical milestone, offering a chance to treat IPF for the first time. Nevertheless substantial gaps in knowledge of this mortal disease remain and attempts to achieve cure of this disease fail up to now.